Skip to main content
SpringerLink
Log in

Plasma Cell Neoplasms (Including Plasma Cell Myeloma)

  • Chapter
  • First Online:
Practical Lymph Node and Bone Marrow Pathology

Part of the book series: Practical Anatomic Pathology ((PAP))

  • 1535 Accesses

Abstract

Plasma cell neoplasms (also known as plasma cell dyscrasias) are diseases in which abnormal plasma cells proliferate and form tumors in the bones or soft tissue of the body. It includes a spectrum of progressively worse diseases, clinically varying from asymptomatic to overtly symptomatic forms. Plasma cell neoplasms include monoclonal gammopathy of undetermined significance (MGUS), plasma cell myeloma, plasmacytoma, monoclonal immunoglobulin deposition diseases, and plasma cell neoplasms with associated paraneoplastic syndrome.

Plasma cell myeloma is one type of plasma cell neoplasms, characterized by proliferation of monoclonal plasma cells producing a monoclonal immunoglobulin (so-called monoclonal gammopathy) and often causing organ damages. There are several morphological variants of malignant plasma cells, ranging from mature plasma cell morphology, immature plasmablastic morphology, small lymphoplasmacytic morphology, to large anaplastic morphology. Plasma cell quantification in bone marrow samples is important in the diagnosis and discrimination of plasma cell myeloma and other types of plasma cell neoplasms.

It is important to reliably distinguish symptomatic multiple myeloma from asymptomatic (smoldering) myeloma and MGUS for the purposes of prognosis and treatment. Immediate treatment is required for patients with multiple myeloma, but not for MGUS and asymptomatic (smoldering) myeloma. Besides evidence of monoclonal gammopathy in serum and/or urine and presence of monoclonal plasma cell population in the bone marrow, symptomatic multiple myeloma is typically associated with end-organ damages such as skeletal destruction with osteolytic lesions and hypercalcemia, anemia, and renal failure. Nonsecretory or nonproducer multiple myeloma and aggressive plasma cell leukemia are rare variants of plasma cell myeloma. The differential diagnosis of multiple myeloma also includes marked reactive bone marrow plasmacytosis, solitary plasmacytoma, plasmablastic lymphoma, lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia, primary amyloidosis, and metastatic carcinoma.

In rare instances plasma cell neoplasm is associated with paraneoplastic syndrome such as POEMS syndrome or TEMPI syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J. WHO classification of tumours of haematopoietic and lymphoid tissues (revised 4th edition). IARC: Lyon; 2017.

    Google Scholar 

  2. Sant M, Allemani C, Tereanu C, De Angelis R, Capocaccia R, Visser O, et al. HAEMACARE Working Group. Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project. Blood. 2010;116(19):3724–34.

    Article  CAS  Google Scholar 

  3. Ocqueteau M, Orfao A, Almeida J, Bladé J, González M, García-Sanz R, et al. Immunophenotypic characterization of plasma cells from monoclonal gammopathy of undetermined significance patients. Implications for the differential diagnosis between MGUS and multiple myeloma. Am J Pathol. 1998;152(6):1655–65.

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Rajkumar SV, Fonseca R, Dispenzieri A, Lacy MQ, Lust JA, Witzig TE, et al. Methods for estimation of bone marrow plasma cell involvement in myeloma: predictive value for response and survival in patients undergoing autologous stem cell transplantation. Am J Hematol. 2001;68(4):269–75.

    Article  CAS  Google Scholar 

  5. Lee N, Moon SY, Lee JH, Park HK, Kong SY, Bang SM, et al. Discrepancies between the percentage of plasma cells in bone marrow aspiration and BM biopsy: impact on the revised IMWG diagnostic criteria of multiple myeloma. Blood Cancer J. 2017;7(2):e530.

    Article  CAS  Google Scholar 

  6. Booth RA, McCudden CR, Balion CM, Blasutig IM, Bouhtiauy I, Rodriguez-Capote K, et al. Candidate recommendations for protein electrophoresis reporting from the Canadian Society of Clinical Chemists Monoclonal Gammopathy Working Group. Clin Biochem. 2018;51:10–20.

    Article  Google Scholar 

  7. Milani P, Palladini G, Merlini G. Serum-free light-chain analysis in diagnosis and management of multiple myeloma and related conditions. Scand J Clin Lab Invest Suppl. 2016;245:S113–8.

    Article  Google Scholar 

  8. Bradwell AR, Carr-Smith HD, Mead GP, Tang LX, Showell PJ, Drayson MT, et al. Highly sensitive, automated immunoassay for immunoglobulin free light chains in serum and urine. Clin Chem. 2001;47:673–80.

    Article  CAS  Google Scholar 

  9. Nowrousian MR, Brandhorst D, Sammet C, Kellert M, Daniels R, Schuett P, et al. Serum free light chain analysis and urine immunofixation electrophoresis in patients with multiple myeloma. Clin Cancer Res. 2005;11(24 Pt 1):8706–14.

    Article  CAS  Google Scholar 

  10. Hutchison CA, Harding S, Hewins P, Mead GP, Townsend J, Bradwell AR, et al. Quantitative assessment of serum and urinary polyclonal free light chains in patients with chronic kidney disease. Clin J Am Soc Nephrol. 2008;3(6):1684–90.

    Article  CAS  Google Scholar 

  11. Sinclair D, Ballantyne F, Shanley S, Caine E, O'Reilly D, Shenkin A. Estimation of paraproteins by immunoturbidimetry and electrophoresis followed by scanning densitometry. Ann Clin Biochem. 1990;27(Pt 4):335–7.

    Article  Google Scholar 

  12. Jacobs JFM, Haagen IA, Lodder A, van der Kroft C, de Kat Angelino CM, Croockewit S, et al. Analytical validation of the Hevylite assays for M-protein quantification. Clin Chem Lab Med. 2018;56(7):1169–75.

    Article  CAS  Google Scholar 

  13. Kyle RA, Gertz MA, Witzig TE, Lust JA, Lacy MQ, Dispenzieri A, et al. Review of 1027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc. 2003;78(1):21–33.

    Article  Google Scholar 

  14. Smith FB, Elnawawi A. A counting strategy for estimating plasma cell number in CD138-stained bone marrow core biopsy sections. Ann Clin Lab Sci. 2008;38(2):138–42.

    PubMed  Google Scholar 

  15. Jelinek T, Bezdekova R, Zatopkova M, Burgos L, Simicek M, Sevcikova T, et al. Current applications of multiparameter flow cytometry in plasma cell disorders. Blood Cancer J. 2017;7(10):e617.

    Article  CAS  Google Scholar 

  16. Nadav L, Katz BZ, Baron S, Yossipov L, Polliack A, Deutsch V, et al. Diverse niches within multiple myeloma bone marrow aspirates affect plasma cell enumeration. Br J Haematol. 2006;133(5):530–2.

    Article  Google Scholar 

  17. Robillard N, Wuillème S, Moreau P, Béné MC. Immunophenotype of normal and myelomatous plasma-cell subsets. Front Immunol. 2014;5:137.

    Article  Google Scholar 

  18. Flores-Montero J, de Tute R, Paiva B, Perez JJ, Böttcher S, Wind H, et al. Immunophenotype of normal vs. myeloma plasma cells: toward antibody panel specifications for MRD detection in multiple myeloma. Cytometry B Clin Cytom. 2016;90(1):61–72.

    Article  CAS  Google Scholar 

  19. Gastinne T, Leleu X, Duhamel A, Moreau AS, Franck G, Andrieux J, et al. Intergroupe francophone du Myelome (IFM). Plasma cell growth fraction using Ki-67 antigen expression identifies a subgroup of multiple myeloma patients displaying short survival within the ISS stage I. Eur J Haematol. 2007;79(4):297–304.

    Article  CAS  Google Scholar 

  20. Soh KT, Tario JD Jr, Wallace PK. Diagnosis of plasma cell dyscrasias and monitoring of minimal residual disease by multiparametric flow cytometry. Clin Lab Med. 2017;37(4):821–53.

    Article  Google Scholar 

  21. Bai Y, Orfao A, Chim CS. Molecular detection of minimal residual disease in multiple myeloma. Br J Haematol. 2018;181(1):11–26.

    Article  CAS  Google Scholar 

  22. Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15(12):e538–48.

    Article  Google Scholar 

  23. Rajkumar SV, Landgren O, Mateos MV. Smoldering multiple myeloma. Blood. 2015;125(20):3069–75.

    Article  CAS  Google Scholar 

  24. Larson D, Kyle RA, Rajkumar SV. Prevalence and monitoring of oligosecretory myeloma. N Engl J Med. 2012;367(6):580–1.

    Article  CAS  Google Scholar 

  25. Jelinek T, Kryukov F, Rihova L, Hajek R. Plasma cell leukemia: from biology to treatment. Eur J Haematol. 2015;95(1):16–26.

    Article  Google Scholar 

  26. Caers J, Paiva B, Zamagni E, Leleu X, Bladé J, Kristinsson SY, et al. Diagnosis, treatment, and response assessment in solitary plasmacytoma: updated recommendations from a European Expert Panel. J Hematol Oncol. 2018;11(1):10.

    Article  CAS  Google Scholar 

  27. Chan NC, Chan NP. Recurrent cytogenetic abnormalities in multiple myeloma. Methods Mol Biol. 2017;1541:295–302.

    Article  CAS  Google Scholar 

  28. Joseph NS, Gentili S, Kaufman JL, Lonial S, Nooka AK. High-risk multiple myeloma: definition and management. Clin Lymphoma Myeloma Leuk. 2017;17S:S80–7.

    Article  Google Scholar 

  29. Fonseca R, Bergsagel PL, Drach J, Shaughnessy J, Gutierrez N, Stewart AK, et al. International Myeloma Working Group. International Myeloma Working Group molecular classification of multiple myeloma: spotlight review. Leukemia. 2009;23(12):2210–21.

    Article  CAS  Google Scholar 

  30. Chesi M, Bargsagel PL. Molecular pathogenesis of multiple myeloma: basic and clinical updates. Int J Haematol. 2013;97(3):313–23.

    Article  CAS  Google Scholar 

  31. Czuchlewski DR, Brackney M, Ewers C, Manna J, Fekrazad MH, Martinez A, et al. Clinicopathologic features of agranulocytosis in the setting of levamisole-tainted cocaine. Am J Clin Pathol. 2010;133(3):466–72.

    Article  Google Scholar 

  32. Hassoun H, Roshal M, Sabari J, Nguyen J, Gao Q, Devlin SM, et al. Immunophenotypic evidence for reactive polyclonal marrow plasmacytosis in multiple myeloma patients receiving lenalidomide maintenance. Leuk Lymphoma. 2017;58(12):2962–5.

    Article  Google Scholar 

  33. Ioannou MG, Stathakis E, Lazaris AC, Papathomas T, Tsiambas E, Koukoulis GK. Immunohistochemical evaluation of 95 bone marrow reactive plasmacytoses. Pathol Oncol Res. 2009;15(1):25–9.

    Article  Google Scholar 

  34. Mansour AT, Shandiz AE, Zimmerman MK, Roth TD, Zhou J. Concomitant lymphoplasmacytic lymphoma and plasma cell myeloma, a diagnostic challenge. Am J Blood Res. 2017;7(2):10–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  35. Elyamany G, Al Mussaed E, Alzahrani AM. Plasmablastic lymphoma: a review of current knowledge and future directions. Adv Hematol. 2015;2015:315289.

    Article  Google Scholar 

  36. Jaccard A. POEMS syndrome: therapeutic options. Hematol Oncol Clin North Am. 2018;32(1):141–51.

    Article  Google Scholar 

  37. Rosado FG, Oliveira JL, Sohani AR, Schroyens W, Sykes DB, Kenderian SS, et al. Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist. Mod Pathol. 2015;28(3):367–72.

    Article  Google Scholar 

  38. Kumar S, Paiva B, Anderson KC, Durie B, Landgren O, Moreau P, et al. International myeloma working group consensus criteria for response and minimal residual disease assessment in multiple myeloma. Lancet Oncol. 2016;17(8):e328–46.

    Article  Google Scholar 

  39. Rajkumar SV, Harousseau JL, Durie B, Anderson KC, Dimopoulos M, Kyle R, et al. International Myeloma Workshop Consensus Panel 1. Consensus recommendations for the uniform reporting of clinical trials: report of the International Myeloma Workshop Consensus Panel 1. Blood. 2011;117(18):4691–5.

    Article  CAS  Google Scholar 

  40. Ravi P, Kumar SK, Cerhan JR, Maurer MJ, Dingli D, Ansell SM, et al. Defining cure in multiple myeloma: a comparative study of outcomes of young individuals with myeloma and curable hematologic malignancies. Blood Cancer J. 2018;8(3):26.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Laboratory Medicine, University of California San Francisco and San Francisco VA Healthcare System, San Francisco, CA, USA

    Chuanyi Mark Lu

Authors
  1. Chuanyi Mark Lu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Chuanyi Mark Lu .

Editor information

Editors and Affiliations

  1. Department of Pathology, Duke University School of Medicine and Duke Health System, Durham, NC, USA

    Endi Wang

  2. Department of Pathology, Duke University School of Medicine and Duke Health System, Durham, NC, USA

    Anand Shreeram Lagoo

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Lu, C.M. (2020). Plasma Cell Neoplasms (Including Plasma Cell Myeloma). In: Wang, E., Lagoo, A.S. (eds) Practical Lymph Node and Bone Marrow Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-32189-5_26

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-32189-5_26

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-32188-8

  • Online ISBN: 978-3-030-32189-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation